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FIGURE: 1 / 10
Please note: We are reviewing Western blot images included in the antibody testing data in our catalog, including those provided by third parties. Unless expressly labeled or annotated as “raw-unedited”, Western blot images included in the antibody testing data in our catalog may have been edited, optimized or otherwise adjusted for presentation.
Recommended positive controls: H1299, HepG2, mouse liver, rat liver.
Predicted reactivity: Mouse (95%), Rat (95%), Zebrafish (82%), Xenopus laevis (88%), Chicken (84%), Rhesus Monkey (100%), Chimpanzee (100%), Bovine (96%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; BCKAD E2; BCKAD-E2; BCKADE2; BCOADC-E2; Branched chain 2-oxo-acid dehydrogenase complex component E2; Branched-chain alpha-keto acid dehydrogenase complex component E2; branched-chain alpha-ketoacid dehydrogenase, E2 subunit; Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex; Dihydrolipoamide branched chain transacylase; dihydrolipoyl transacylase; Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase; Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial; MGC9061; part of the BCKAD complex
Gene Aliases: BCATE2; BCKDHE2; D3Wsu60e; DBT
UniProt ID: (Mouse) P53395
Entrez Gene ID: (Mouse) 13171, (Rat) 29611
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